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Article

Acanthocyte Sedimentation Rate as a Diagnostic Biomarker for Neuroacanthocytosis Syndromes: Experimental Evidence and Physical Justification

1
Experimental Physics, Saarland University, 66123 Saarbruecken, Germany
2
Translational Neurodegeneration Section “Albrecht-Kossel”, Department of Neurology, University Medical Center Rostock, University of Rostock, 18051 Rostock, Germany
3
Neurodegenerative Diseases, Department of Neurology, Technische Universität Dresden, 01062 Dresden, Germany
4
Theoretical Medicine and Biosciences, Saarland University, 66424 Homburg, Germany
5
Laboratoire Interdisciplinaire de Physique, UMR 5588, 38402 Saint Martin d’Hères, France
6
Institute for Clinical and Experimental Surgery, Saarland University, 66424 Homburg, Germany
7
Institute of Biological Information Processing and Institute for Advanced Simulation, Forschungszentrum Jülich, 52425 Jülich, Germany
8
DZNE, German Center for Neurodegenerative Diseases, Research Site Rostock/Greifswald, 18051 Rostock, Germany
9
Center for Transdisciplinary Neurosciences Rostock (CTNR), University Medical Center Rostock, University of Rostock, 18051 Rostock, Germany
10
Neurologische Klinik und Poliklinik, Ludwig-Maximilians-Universität, 81366 Munich, Germany
11
Physics and Materials Science Research Unit, University of Luxembourg, 1511 Luxembourg, Luxembourg
*
Authors to whom correspondence should be addressed.
These authors contributed equally to this work.
Academic Editor: Saghi Ghaffari
Cells 2021, 10(4), 788; https://doi.org/10.3390/cells10040788
Received: 26 February 2021 / Revised: 26 March 2021 / Accepted: 30 March 2021 / Published: 2 April 2021
(This article belongs to the Special Issue Advances in Red Blood Cells Research)
(1) Background: Chorea-acanthocytosis and McLeod syndrome are the core diseases among the group of rare neurodegenerative disorders called neuroacanthocytosis syndromes (NASs). NAS patients have a variable number of irregularly spiky erythrocytes, so-called acanthocytes. Their detection is a crucial but error-prone parameter in the diagnosis of NASs, often leading to misdiagnoses. (2) Methods: We measured the standard Westergren erythrocyte sedimentation rate (ESR) of various blood samples from NAS patients and healthy controls. Furthermore, we manipulated the ESR by swapping the erythrocytes and plasma of different individuals, as well as replacing plasma with dextran. These measurements were complemented by clinical laboratory data and single-cell adhesion force measurements. Additionally, we followed theoretical modeling approaches. (3) Results: We show that the acanthocyte sedimentation rate (ASR) with a two-hour read-out is significantly prolonged in chorea-acanthocytosis and McLeod syndrome without overlap compared to the ESR of the controls. Mechanistically, through modern colloidal physics, we show that acanthocyte aggregation and plasma fibrinogen levels slow down the sedimentation. Moreover, the inverse of ASR correlates with the number of acanthocytes (R2=0.61, p=0.004). (4) Conclusions: The ASR/ESR is a clear, robust and easily obtainable diagnostic marker. Independently of NASs, we also regard this study as a hallmark of the physical view of erythrocyte sedimentation by describing anticoagulated blood in stasis as a percolating gel, allowing the application of colloidal physics theory. View Full-Text
Keywords: neuroacanthocytosis; erythrocyte sedimentation rate (ESR); diagnosis neuroacanthocytosis; erythrocyte sedimentation rate (ESR); diagnosis
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MDPI and ACS Style

Darras, A.; Peikert, K.; Rabe, A.; Yaya, F.; Simionato, G.; John, T.; Dasanna, A.K.; Buvalyy, S.; Geisel, J.; Hermann, A.; Fedosov, D.A.; Danek, A.; Wagner, C.; Kaestner, L. Acanthocyte Sedimentation Rate as a Diagnostic Biomarker for Neuroacanthocytosis Syndromes: Experimental Evidence and Physical Justification. Cells 2021, 10, 788. https://doi.org/10.3390/cells10040788

AMA Style

Darras A, Peikert K, Rabe A, Yaya F, Simionato G, John T, Dasanna AK, Buvalyy S, Geisel J, Hermann A, Fedosov DA, Danek A, Wagner C, Kaestner L. Acanthocyte Sedimentation Rate as a Diagnostic Biomarker for Neuroacanthocytosis Syndromes: Experimental Evidence and Physical Justification. Cells. 2021; 10(4):788. https://doi.org/10.3390/cells10040788

Chicago/Turabian Style

Darras, Alexis, Kevin Peikert, Antonia Rabe, François Yaya, Greta Simionato, Thomas John, Anil K. Dasanna, Semen Buvalyy, Jürgen Geisel, Andreas Hermann, Dmitry A. Fedosov, Adrian Danek, Christian Wagner, and Lars Kaestner. 2021. "Acanthocyte Sedimentation Rate as a Diagnostic Biomarker for Neuroacanthocytosis Syndromes: Experimental Evidence and Physical Justification" Cells 10, no. 4: 788. https://doi.org/10.3390/cells10040788

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